ReviewDigestive diseases and sciences2010
Primary intestinal lymphangiectasia: four case reports and a review of the literature.
Review in Digestive diseases and sciences, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 91 citations in OpenAlex.
- Peripheral Lymphatic Shunts for Protein-Losing Enteropathy in a Child with THSD1 Mutation: A Case Report.Archives of plastic surgery · 2026Article
- Primary Intestinal Lymphangiectasia as a cause of secondary combined immunodeficiency: case study and literature review.Clinical immunology communications · 2026Article
- Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.Journal of pediatric gastroenterology and nutrition · 2026Article
- Beyond Drainage: How Lymphatic Vessels Instruct Immune Cell Fate.Journal of inflammation research · 2026Review
- Case Report: Six infants with primary intestinal lymphangiectasia: clinical manifestations of refractory cases and preliminary stratified clinical management experience.Frontiers in pediatrics · 2026Article
- Clinical characterization of primary intestinal lymphangiectasia in infants and young children: a single-center retrospective analysis in China.Translational pediatrics · 2025Article
- Primary intestinal lymphangiectasia: French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins).Orphanet journal of rare diseases · 2025Review
- Elusive Edematous Primary Intestinal Lymphangiectasia in an Adult Patient Presenting With New Onset of Protein-Losing Enteropathy.ACG case reports journal · 2025Article
- Importance of scattered white spots detected in the duodenum and their relationship with zonulin.Pakistan journal of medical sciences · 2024Article
- Misty mesentery, ascites, and bowel wall thickening in a child: Diagnostic clue for intestinal lymphangiectasia.Radiology case reports · 2023Article
- Small intestinal mucosal abnormalities using video capsule endoscopy in intestinal lymphangiectasia.Orphanet journal of rare diseases · 2023Article
- Article
- Pathophysiology, Diagnosis, and Management of Canine Intestinal Lymphangiectasia: A Comparative Review.Animals : an open access journal from MDPI · 2022Review
- Primary intestinal lymphangiectasia presenting as limb convulsions: A case report.World journal of clinical cases · 2022Article
- Endoscopic classification and pathological features of primary intestinal lymphangiectasia.World journal of gastroenterology · 2022Article
- Pharmacokinetics and toxicity evaluation following oral exposure to bisphenol F.Archives of toxicology · 2022Article
- Novel mutation causing congenital disorder of glycosylation in a child with recurrent anasarca.BMJ case reports · 2022Article
- A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma.BMC gastroenterology · 2021Article
- Complicated primary intestinal lymphangiectasia (Waldmann's disease) in a child successfully treated with octreotide: A case report from a low-resource setting.Annals of medicine and surgery (2012) · 2021Article
- Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review.BMC gastroenterology · 2021Review
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPrimary intestinal lymphangiectasia (PIL) is a rare digestive disease and most articles on this condition are isolated case reports.
aimsOur purpose is to investigate the clinical characteristics, therapeutic management, and outcome of PIL through case studies.
methodsWe conducted a retrospective analysis and obtained detailed clinical information for four PIL patients treated at our institution. A MEDLINE database search was also performed using the search term "intestinal lymphangiectasia" and all pertinent literature was carefully reviewed.
resultsFour children treated in our department showed elevated IgE and a good response to diet intervention. After reviewing the literature, we conducted statistical analysis on the basis of all the cases, with a total of 84 cases. Thirty-eight cases have been reported with diet treatment, 24 (63%) of whom showed apparent improvement in clinical symptoms and laboratory parameters. Four cases (5%) had a malignant transformation of lymphoma, and the average time from PIL onset to lymphoma diagnosis was 31 years (range, 19-45 years). No difference was observed regarding the presence of major clinical manifestations among children and adults. Diet intervention in children was more effective than that in adults.
conclusionsDiet intervention is the cornerstone of PIL medical management, which was found to be more effective in children than in adults. Early diagnosis and treatment of IL is of great importance for effective diet therapy. An elevated IgE level should be monitored periodically since it could be an indication of malignant transformation-lymphoma.
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